Objective To investigate the influence of different timing of surgery on patients with poor-grade intracranial aneurysm. Methods The clinical data of 86 patients with aneurysmal subarachnoid hemorrhage (Hunt-Hess gradeⅣ toⅤ)were analyzed retrospectively. The patients were divided into an ultra-early treatment group (the operative time within 24 h after onset;n=40),an early treatment group, (24 to 72 h after onset;n=27),and middle and late treatment group (>72 h after onset;n=19)according to the different timing of surgery. The prognosis of patients at 6 months after procedure was evaluated with the modified Rankin Scale (mRS)scores. The differences of the rate of good prognosis (mRS 0 to 2)and mortality in patients of the 3 groups were compared. Results (1 )The ratios of good prognosis in the ultra-early treatment group,early treatment group,and middle and late treatment group were 55. 0%(n=22),33. 3%(n=9),and 21. 1%(n=4),respectively. There were significant differences (P<0. 05). The mortality rate of the ultra-early treatment group (7. 5%)was lower than that of the early treatment group (25. 9%) or middle and late treatment group (42. 1%). There were significant differences (P<0. 05). (2)There were significant differences in the rate of good prognosis in different timing of surgery in patients with gradeⅣ(P<0. 05);there was no significant difference in the rate of good prognosis in patients with grade Ⅴ(P>0. 05). The mortality ratios in patients of the 3 groups were 2/18,4/11,and 6/11,respectively. There were significant differences (P<0. 05). Conclusion Ultra-early operation may benefit part of the patients with poor-grade aneurysm,particularly in patients with gradeⅣ. As for the patients with grade Ⅴ, ultra-early operation may help to reduce short-term mortality rate,however,the rate of vegetative state is high.

OBJECTIVE: To analyze the clinical and molecular biological characteristics of a neonate with myeloid proliferation related to Down syndrome (DS). METHODS: The neonate, who was suspected for Down syndrome, was analyzed in terms of clinical feature, peripheral blood cell morphology, fluorescence in situ hybridization (FISH), immunological classification and other laboratory tests. On hundred and fourteen leukemia-related genes were subjected to next-generation sequencing (NGS). RESULTS: Laboratory test revealed obvious abnormal liver function and coagulation function, anemia, and extreme leukocytosis. Cell smear indicated significantly increased progenitor cells, which conformed to proliferation of megakaryocytes. FISH showed trisomy 21. By NGS, c.220+dupT, a novel mutation, was identified in exon 2 of the GATA1 gene, which encodes a N-terminal activation domain and has a frequency of 95.8%. No mutation was identified among the remaining 113 genes. CONCLUSION The neonate had DS and GATA1 gene mutation. High percentage of circulating blasts should be considered as transient myelodysplasia but not congenital leukemia.
Down Syndrome ; genetics ; GATA1 Transcription Factor ; genetics ; Humans ; In Situ Hybridization, Fluorescence ; Infant, Newborn ; Mutation ; Trisomy

Objective To investigate the application value of digital subtraction angiography ( DSA) combined with volume rendering technique ( VRT) in intracranial aneurysm clipping. Methods From January to July 2016,19 consecutive patients with intracranial aneurysm admitted to the Department of Neurosurgery,Northern Jiangsu People′s Hospital were enrolled retrospectively. All patients underwent craniotomy clipping immediately after clipping,DSA was performed and VRT was use to process the related images. For those with poor clipping effect, angiography was performed again after the adjustment of the aneurysm clips,until angiography confirmed that the clipping was satisfactory. Good treatment was defined as no recurrence of aneurysms and the Glasgow outcome scale of 4-5 . Results Nineteen patients had 26 aneurysms. They were all clipped completely observed under the microscope. Confirmed by angiography, 18 aneurysms were clipped completely for the first time,1 apical aneurysm of basilar artery and its contralateral posterior cerebral artery were clipped with a residual aneurysm neck,the contralateral A2 artery was clipped in 1 anterior communicating artery aneurysm, an anterior choroidal artery was clipped completetly in 1 posterior communicating aneurysm,3 aneurysms had residual necks, the upper stem artery was stenosis after 1 middle cerebral artery bifurcation aneurysm was clipped,the anterior communicating artery and the origin of contralateral A2 was stenotic after one anterior communicating artery aneurysm being clipped.Except for 2 aneurysms ruptured without adjustment during the operation,the clipping of other aneurysms was ideal after adjustment. In 2 patients with severe cerebral vasospasm,1 was relieved after a slow transcatheter injection of papaverine,1 was relieved after putting papaverine-wetted cotton piece on the site of vasospasm. The intraoperative DSA and VRT image processing time was 30-100 min. No complications associated with angiography occurred. The patients were followed up for 3-16 months,computed tomography angiography was showed no aneurysm recurrence and vascular stenosis,there were 1 case with hemiplegia and 18 cases with good recovery. Conclusions Intraoperative DSA combined with VRT can help to observe the clipping effect in real time and adjust the aneurysm clips. It can reduce the residual aneurysm neck,parent artery,and peritumoral vascular occlusion,thus improving the operation effect and reducing the disability and mortality.

Objective:To investigate the safety and effectiveness of emergency one-stop hybrid operation in ruptured and hemorrhagic high-grade brain arteriovenous malformations (BAVMs).Methods:Twelve patients with ruptured and hemorrhagic high-grade BAVMs who underwent emergency one-stop hybrid operation were chosen. The clinical data and efficacy of these patients were analyzed retrospectively.Results:All patients completed surgery successfully. Embolization combined with BAVMs resection was performed in 4 patients, BAVMs resection under digital subtraction angiography (DSA) was performed in 4 patients, embolization combined with balloon-assisted BAVMs resection was performed in 1 patient, balloon-assisted BAVMs resection under DSA was performed in 1 patient, embolization combined with BAVMs resection and aneurysm clipping was performed in 1 patient, and BAVMs resection combined with aneurysm clipping under DSA was performed in 1 patient. Intraoperative DSA showed residual vascular malformation in 1 patient and complete resection in other 11 patients. No surgical complications occurred. Two patients presented symptoms of delayed epilepsy after discharge. Six months after operation, 8 patients had good prognosis (Glasgow Outcome Scale [GOS] scores≥4), 4 had poor prognosis (GOS scores of 1-3), and no death occurred. CTA or DSA in all patients 6 months after operation found no residual or recurrent vascular lesions.Conclusion:Emergency one-stop hybrid operation has high complete occlusion rate and low surgical complication incidence in ruptured and hemorrhagic high-grade BAVMs, which is worthy of clinical application.